Krishnan Jayashree, Frizzera Glauco
Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY, USA.
Semin Diagn Pathol. 2003 May;20(2):94-104. doi: 10.1016/s0740-2570(03)00014-5.
This short review is dedicated to a precise pathologic characterization of 2 uncommon and poorly defined lesions of the spleen and to their distinction from histologically similar processes. Splenic hamartoma represents an abnormally formed red pulp and is characterized by the presence of sinus-like structures lined by CD8(+) endothelia. The great variety of its morphologic appearances may result from the preponderant growth of one or another of the several components of the red pulp, ie, CD34(+) capillaries, myoid cells and macrophages. Therefore, it is proposed that "cord capillary hemangioma," myoid angio-endothelioma, and histiocyte-rich tumors are part of the spectrum of splenic hamartoma. Inflammatory pseudotumor (IPT) of the spleen is a reactive lesion, probably of multiple etiologies, characterized by a mixture of inflammatory cells and a minor, disorganized component of spindle cells. The latter include fibroblasts, SMA(+) myofibroblasts, and CD68(+) spindled histiocytes, establishing a close similarity with the IPT of the lymph node. This benign process needs to be distinguished from 2 others that have a predominant spindle cell component arranged in parallel bundles: the IPT-like follicular dendritic cell tumor, which is consistently associated with Epstein-Barr Virus; and the inflammatory myofibroblastic tumor, also often Epstein-Barr Virus-related and similar to those of the soft tissues, lung and other organs. These 2 lesions are neoplastic and therefore have a potentially worse prognosis than IPT.
本简短综述致力于对脾脏2种罕见且定义不清的病变进行精确的病理特征描述,并将它们与组织学上相似的病变相鉴别。脾错构瘤表现为异常形成的红髓,其特征是存在由CD8(+)内皮细胞衬里的窦样结构。其形态学表现的多样性可能源于红髓的几种成分(即CD34(+)毛细血管、肌样细胞和巨噬细胞)中一种或另一种成分的优势生长。因此,有人提出“索状毛细血管血管瘤”、肌样血管内皮瘤和富含组织细胞的肿瘤是脾错构瘤谱系的一部分。脾脏炎性假瘤(IPT)是一种反应性病变,可能由多种病因引起,其特征是炎性细胞混合以及少量排列紊乱的梭形细胞成分。后者包括成纤维细胞、平滑肌肌动蛋白(SMA)(+)肌成纤维细胞和CD68(+)梭形组织细胞,这与淋巴结的IPT非常相似。这个良性过程需要与另外2种以平行束状排列的主要梭形细胞成分为特征的病变相鉴别:IPT样滤泡树突状细胞瘤,它始终与爱泼斯坦-巴尔病毒相关;以及炎性肌纤维母细胞瘤,它也常与爱泼斯坦-巴尔病毒相关,并且与软组织、肺和其他器官的炎性肌纤维母细胞瘤相似。这2种病变是肿瘤性的,因此预后可能比IPT更差。